Maria Ana Serrado1, Mariana Horta2, Teresa Margarida Cunha2. 1. Radiology Department, Hospital Central do Funchal, Funchal, Portugal. 2. Radiology Department, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal.
Abstract
Vulvar carcinoma is an uncommon tumor that predominantly affects postmenopausal women. Currently, there is no screening procedure for vulvar carcinoma; in most cases, it is diagnosed only when symptoms appear. The most widely used staging system is that developed by the International Federation of Gynecology and Obstetrics. Lymph node status is the most important prognostic factor. We searched the PubMed/Medline database to identify relevant English-language articles on vulvar cancer, with a special focus on its imaging evaluation. Magnetic resonance imaging is useful for local and nodal staging, as well as facilitating the planning of surgical interventions and radiotherapy. Computed tomography or positron-emission tomography/computed tomography can play an important role in nodal and distant disease assessment, whereas ultrasound is often used for image-guided biopsies. Imaging is pivotal for staging and treatment planning in vulvar carcinoma.
Vulvar carcinoma is an uncommon tumor that predominantly affects postmenopausal women. Currently, there is no screening procedure for vulvar carcinoma; in most cases, it is diagnosed only when symptoms appear. The most widely used staging system is that developed by the International Federation of Gynecology and Obstetrics. Lymph node status is the most important prognostic factor. We searched the PubMed/Medline database to identify relevant English-language articles on vulvar cancer, with a special focus on its imaging evaluation. Magnetic resonance imaging is useful for local and nodal staging, as well as facilitating the planning of surgical interventions and radiotherapy. Computed tomography or positron-emission tomography/computed tomography can play an important role in nodal and distant disease assessment, whereas ultrasound is often used for image-guided biopsies. Imaging is pivotal for staging and treatment planning in vulvar carcinoma.
Carcinoma of the vulva is an uncommon tumor, accounting for only approximately 4% of
gynecologic malignancies. In the United States, there were an estimated 6190 new
cases of vulvar cancer and 1200 deaths associated with the disease in
2018([1]).
It predominantly affects postmenopausal women, its incidence increasing with
age([2]).
Currently, there is no screening procedure for vulvar carcinoma. However, patients
with a history of cervical or vaginal cancer, vulvar intraepithelial neoplasia, or
lichen sclerosus should be kept under regular surveillance([2]).In most cases, vulvar carcinoma is symptomatic, presenting as a painful vulvar lump
or ulcer, pruritus, bleeding, and discharge. When there are metastases to the
inguinal lymph nodes, a lump in the groin can be reported. Occasionally, vulvar
carcinoma is asymptomatic([2]). It is confined to the primary site in 59% of the
cases, spreads to regional lymph nodes in 30%, and causes distant disease in 6%. The
5-year relative survival rate is linked to the stage at diagnosis. The 5-year
survival rate is 86% for localized disease, 53% for regional spread, and 19% for
distant disease([3]). The most important prognostic factor is lymph node
status. Survival is impaired in patients with positive lymph nodes; 88% of
node-negative patients are free of disease within two years. Of the patients with
one, two, or more than two positive lymph nodes, 60%, 43%, and 29%, respectively,
are free of disease within two years([4]). The management of vulvar carcinoma must
therefore be individualized.The aim of this study was to review the imaging aspects of vulvar carcinoma, its
staging, and the influence of imaging findings on treatment planning. We searched
the PubMed/Medline database, using the following search terms: vulvar cancer; vulvar
carcinoma; gynecologic malignancies; ultrasound; computed tomography (CT); magnetic
resonance imaging (MRI); and positron-emission tomography/computed tomography
(PET/CT). We selected relevant English-language papers on vulvar cancer, with a
special focus on its imaging evaluation.
VULVAR ANATOMY, VULVAR CARCINOMA, AND TUMOR SPREAD
The vulva is bounded anteriorly by the symphysis pubis, posteriorly by the anal
sphincter, superiorly by the urogenital diaphragm, and laterally by the ischial
tuberosity([5],[6]). As depicted in Figure
1, it is composed of superficial structures (including the labia majora
and minora), as well as the vestibule of the vagina, opening of the urethra,
clitoral glans, and clitoral body; the bulbospongiosus and ischiocavernosus muscles;
and the vestibular bulbs and the crura of the clitoris. The clitoris is composed of
the glans, body, and crura (right and left), which are in turn composed of the
corpora cavernosa and covered by the ischiocavernosus muscle. The vestibular bulb is
a venous plexus surrounded by fascia and covered by the bulbospongiosus
muscle([5]).
Figure 1
Schematic representation of the superficial and deep structures of the
vulva.
Schematic representation of the superficial and deep structures of the
vulva.The vulva is irrigated via branches of the external and internal pudendal
arteries([6]). The venous drainage is mainly via the (external and
internal) pudendal veins, perineal vein, and deep posterior vein of the
clitoris([5]). The lymphatic drainage from the vulva is primarily
via the superficial inguinal nodes, although the deep inguinal nodes are also
involved([6]). The superficial inguinal nodes are located deeper
than the inguinal ring and can be divided into three groups: medial, intermediate,
and lateral. The deep inguinal nodes lie medial to the femoral
vein([7]). The subsequent drainage from those nodes is via the
external iliac and pelvic nodes([5],[8],[9]).Vulvar carcinomas can be divided into those that are related to human papillomavirus
infection and those that are not. The former lead to squamous cell carcinoma in
younger women and account for 20% of all cases of invasive disease. The latter lead
to squamous cell carcinoma in older patients and account for 80% of all cases of
invasive disease([10]).Nearly 70% of vulvar carcinomas involve the labia majora and minora. In 15-20% of
cases, the lesion involves the clitoris. In approximately 10% of cases, the lesion
is so extensive that the primary location cannot be identified. Multifocal lesions
occur in 5% of cases([5]).Lateral vulvar carcinomas drain to the ipsilateral inguinal lymph nodes. Because of
the rich lymphatic network across the midline of the vulva, midline lesions and
lesions within 1 cm of the midline can drain bilaterally([5],[7],[8]). The deep inguinal lymph
nodes can be infiltrated by tumor cells, although the superficial inguinal lymph
nodes are not([7]). As can be seen in Figure 2, the pelvic lymph nodes are rarely involved unless the
ipsilateral inguinal lymph nodes are as well([5],[7],[8]).
Figure 2
Schematic representation of lymphatic drainage of carcinoma of the
vulva.
Schematic representation of lymphatic drainage of carcinoma of the
vulva.In rare cases, vulvar carcinoma can spread to the pelvic lymph nodes via the internal
pudendal chain and internal iliac chain, as direct drainage from the
midline([5],[8]). If there is obstruction of the typical lymphatic
drainage, the tumor can spread via the subcutaneous and dermal lymphatic systems of
the upper thigh and lower abdomen([5],[8]). If the tumor has invaded the vagina, bladder, or
anus (above the dentate line), it can spread to the obturator or internal iliac
lymph nodes([5]). Distant metastases are rare if the disease is
diagnosed in its early stages([11]).
THE ROLE OF IMAGING
Imaging appearance of the vulva
Due to its excellent soft tissue resolution, MRI is the ideal imaging modality
for assessing vulvar anatomy. The normal vulva has low to intermediate signal
intensity on T1-weighted imaging (T1WI), whereas the signal intensity on
T2-weighted imaging (T2WI) is slightly higher([6]).Even with intravenous contrast agent administration, CT is limited in its ability
to depict the vulva. On CT, the vulva appears as a triangular soft tissue
structure([5]).
Imaging modalities
Ultrasound and ultrasound-guided fine-needle aspiration cytology
The role of ultrasound and fine-needle aspiration cytology (FNAC) in the
evaluation of nodal status in vulvar cancer has been subject of several
studies. When used in isolation, the reported sensitivity of ultrasound
ranges from 76% to 100% and its reported specificity ranges from 69% to
91%([12]-[16]). That wide variation can be attributed
to a variety of factors related to the lymph node in question, including the
short-axis diameter, the ratio of long- to short-axis diameter, an irregular
shape, and the absence of a fatty hilum, as well as the overall attenuation
and peripheral vascularization. It has been suggested that greater
peripheral vascularity on color Doppler, especially on power Doppler, is
indicative of metastatic nodes. A spectral waveform showing a high resistive
index can further strengthen that hypothesis([15]).Moskovic et al.([13]) studied the diagnostic performance of FNAC
in comparison with that of the combination of ultrasound and FNAC. The
authors found that the use of FNAC alone had a sensitivity and specificity
of 58% and 100%, respectively, and that combining ultrasound with FNAC
increased the sensitivity to 83% but reduced the specificity to 82%. In a
subsequent study conducted at the same institution and evaluating the same
parameters, the accuracy of that combination was shown to have improved over
time, reaching a sensitivity of 93% and a specificity of
100%([15]). Consequently, some authors have suggested
that the combination of ultrasound and FNAC can prevent unnecessary inguinal
lymph node dissection([15]). However, the use of ultrasound alone
cannot replace surgical lymph node staging([16]).
MRI
Patient preparation is important for obtaining optimal results with MRI.
Fasting for 4-6 hours before the examination, anti-peristaltic agent
administration, bladder voiding, and vaginal distention with ultrasound gel
may all be considered. The standard MRI protocol for the evaluation of
vulvar lesions includes axial T1-weighted fast spin-echo images with a large
field of view; axial and coronal high-resolution T2-weighted fast spin-echo
images; and sagittal T1WI([7]). T2WI with fat suppression may show the
tumor better than do images without fat suppression([7],[9],[17]), given that
the perineal region is rich in fat, that tumors with intermediate signal
intensity on T2WI (Figure 3A) are more
difficult to visualize, and that the intrinsic signal intensity of the tumor
is associated with necrosis (Figure
3B). Dynamic contrast-enhanced MRI can aid in the evaluation of small
tumors and of involvement of the urethra, anus, and
vagina([7],[9]), as shown in Figure 3C. Diffusion-weighted imaging can also be useful for
delineating primary masses and lymph node metastases([5],[9]).
Figure 3
FIGO stage IB vulvar carcinoma. A: Axial T2WI
showing a right-sided vulvar lesion with intermediate signal
intensity (arrow). B: Axial T2WI with fat
suppression better delineated the tumor (arrow). C:
Contrast-enhanced axial T1WI with fat suppression, showing the
same tumor (arrow). Pathology, at surgery, revealed a 37 mm
tumor with 5 mm of invasion, confirming the diagnosis of stage
IB disease.
FIGO stage IB vulvar carcinoma. A: Axial T2WI
showing a right-sided vulvar lesion with intermediate signal
intensity (arrow). B: Axial T2WI with fat
suppression better delineated the tumor (arrow). C:
Contrast-enhanced axial T1WI with fat suppression, showing the
same tumor (arrow). Pathology, at surgery, revealed a 37 mm
tumor with 5 mm of invasion, confirming the diagnosis of stage
IB disease.The assessment of primary vulvar lesions is best achieved by MRI. Sohaib et
al.([18]) reported that the MRI staging of vulvar
cancers was correct in 70% of cases. Kataoka et al.([17]) studied the
accuracy of unenhanced and contrast-enhanced MRI in determining the size and
stage of vulvar cancers. In the evaluation of lesion size, unenhanced MRI
was found to have an accuracy of 86% for primary vulvar cancers and 72% for
recurrent vulvar cancers, resulting in an overall accuracy of 83%. The use
of contrast did not change the accuracy of MRI in the evaluation of the size
of primary vulvar cancers, although it did increase that accuracy (to 80%)
in recurrent vulvar cancers. For the staging of primary vulvar cancer, the
reported accuracy of unenhanced MRI is 69%, whereas that of
contrast-enhanced MRI is 85%([17]). MRI is also the best imaging method of
determining the involvement of adjacent structures, which is pivotal for the
surgical planning.Some studies have used MRI to evaluate lymph node metastases in vulvar
cancer. Those studies have reported widely varying sensitivities and
specificities, ranging from 40% to 89% and from 81% to 100%,
respectively([17]-[21]). In addition, several criteria have
been proposed as being the most accurate in identifying suspicious lymph
nodes. Although the short-axis diameter is the most commonly used criterion,
its sensitivity is low, ranging from 40% to 50%, depending on the location
of the lymph node and the cut-off value employed([18]).Hawnaur et al.([19]) suggested that the most useful single
criterion is that of lymph node contour irregularity. It has also been
suggested that the ratio of long- to short-axis diameter is a good
quantitative marker for diagnosing lymph node metastases, with a sensitivity
of 85% and a specificity of 81%([17]). Among individual criteria,
lymph node necrosis has been shown to have the highest specificity, although
it has low sensitivity([17]). Bipat et al.([20]) demonstrated
that, in the evaluation of several criteria, such as short-axis diameter,
aspect, margin, and ratio of long- to short-axis diameter, the interobserver
agreement was good (kappa = 0.62). False-positive results can be related to
reactive changes, particularly when the MRI is performed soon after a
diagnostic vulvar biopsy([21]).
CT
In suspected cases of vulvar cancer, CT should be performed with oral,
intravenous, and rectal contrast administration. Images are usually obtained
with 2.5-mm axial slice reconstructions, including the upper thighs to cover
the entire extent of the involvement([7]). However, CT is of limited
usefulness in the evaluation of primary vulvar lesions. It is not routinely
recommended for the detection of inguinal lymph nodes, for which it is
reported to have a sensitivity of 58-60%, a specificity of 75-90%, a
positive predictive value of 38-58%, and a negative predictive value of
75-96%([12],[22]). Preoperative CT has been shown to have
no influence on the treatment strategy([12],[22]). Therefore, CT may play a role
only when there is clinical evidence of diseased inguinal lymph nodes-in
order to assess the proximity of such nodes to the femoral vessels and to
evaluate the pelvic lymph nodes-or when there is surgically proven inguinal
lymph node involvement-in order to plan the postoperative
treatment([12],[22]).Because a short-axis diameter of ≥ 1 cm is the standard criterion to
identify metastatic lymph nodes, metastatic lymph nodes < 1 cm may be
missed on CT([7]). In a study conducted by Lin et
al.([23]), CT and MRI were both found to be
significantly more efficacious than was 18F-fluorodeoxyglucose
PET/CT (18F-FDG PET/CT) in detecting pelvic lymph node
involvement and distant metastases. However, the authors stated that the
high sensitivity and negative predictive value of 18F-FDG PET/CT
can be reassuring after suspicious lymph nodes have been identified on CT or
MRI.
18F-FDG PET/CT
In 18F-FDG PET/CT, the imaging is performed 60-90 min after
intravenous administration of 18F-FDG. The body is scanned, in
the caudocranial direction, from the upper thighs to the skull vertex.
Patients are required to empty their bladder before imaging. The studies are
usually performed on an integrated PET/CT scanner. Unenhanced axial CT is
performed for attenuation correction and diagnosis, thereafter being
reconstructed to match the slice thickness of the PET
images([7]).The accuracy of 18F-FDG PET/CT in detecting primary vulvar lesions
is 100%([24]). However, like CT, it plays a limited role
in the assessment of the extent of the tumor. Several studies have
investigated the role of PET and 18F-FDG PET/CT in the evaluation
of inguinal lymph nodes, reporting a wide range of diagnostic performance
levels([23]-[27]). Sensitivities ranging from 50% to 100%
and specificities ranging from 67% to 100% have been reported. The majority
of such studies state that 18F-FDG PET/CT is not indicated in
nodal staging, because of its low sensitivity([25]-[27]). The ideal
cut-off of maximum standardized uptake value (SUVmax) is not well
established in the literature. The reported range for the mean
SUVmax of metastatic inguinal lymph nodes is from 6.1 to
11.0([23],[26]). 18F-FDG PET/CT has limited
value in the evaluation of lymph node metastases < 5 mm. It can also
produce false-negative results in necrotic lymph nodes and false-positive
results in inflammatory lymph nodes([7],[24]). The 100% negative predictive
value of 18F-FDG PET/CT can be supportive when pelvic lymph node
involvement or distant metastases are suspected on CT or
MRI([23]).
STAGING SYSTEM, IMAGING FINDINGS, AND TREATMENT
The most widely used staging system is that developed by the International Federation
of Gynecology and Obstetrics (FIGO), which was revised in 2009([28]). As previously
reported([2],[29]), the 2009 revision of the FIGO staging system
corrected several previous limitations, in the following ways (Table 1): by combining stages IB and II into a
new stage IB; by dividing stage III into the new stages II and III; by stating that
all cases with positive inguinal lymph nodes should now be classified as stage III;
and by no longer considering bilateral inguinal disease. Those changes have been
validated in several studies([30]-[32]).
Table 1
FIGO staging for carcinoma of the vulva, 2009 revision.
Stage
Definition
I
Tumor confined to the vulva.
IA
Lesions ≤ 2 cm in size, confined to the vulva or
perineum and with stromal invasion ≤ 1 mm, no nodal
metastasis.
IB
Lesions > 2 cm or with stromal invasion < 1 mm,
confined to the vulva or perineum, with negative lymph nodes.
II
Tumor of any size extending to adjacent perineal
structures (lower 1/3 of the urethra, lower 1/3 of the vagina, or
anus) with negative lymph nodes.
III
Tumor of any size, with or without extension to
adjacent perineal structures (lower 1/3 of the urethra, lower 1/3 of
the vagina, or anus), with positive inguinofemoral lymph nodes.
IIIA
i) 1 Lymph node metastasis ≥ 5 mm; or ii) 1-2
lymph node metastases < 5 mm.
IIIB
i) ≥ 2 Lymph node metastases ≥ 5 mm; or
ii) ≥ 3 lymph node metastases < 5 mm.
IIIC
Positive lymph nodes with extracapsular spread.
IV
Tumor invades other regional structures (upper 2/3 of
the urethra or upper 2/3 of the vagina) or distant structures.
IVA
i) Tumor invasion of the upper urethra, vaginal mucosa,
bladder mucosa, rectal mucosa, or pelvic bone; or (ii) fixed or
ulcerated inguinofemoral lymph nodes.
IVB
Distant metastasis to any site, including the pelvic
lymph nodes.
FIGO staging for carcinoma of the vulva, 2009 revision.
Stages I and II-localized disease
Stage IA disease is defined as a tumor ≤ 2 cm with stromal invasion
≤ 1 mm. Stage IB is defined as a tumor > 2 cm with stromal invasion
> 1 mm. In both stages, there is no nodal involvement, invasion of adjacent
structures, or distant disease. Stage II is defined as a tumor of any size with
invasion of adjacent perineal structures.
Imaging findings
The role of imaging is limited in stages IA, IB, and II. Vulvar carcinoma is
depicted as a solid mass with low signal intensity on T1WI and intermediate
to high signal intensity on T2WI. On dynamic contrast-enhanced sequences
(Figure 3), there is contrast
enhancement in the early arterial phase([7]). Disruption of
the target appearance of the urethra on T2WI and interruption of the low
signal intensity on T2WI of the vaginal wall (Figure 4A) or anal sphincter by an intermediate to high signal
intensity tumor on T2WI (Figure 4B) are
suggestive of invasion([9]). On CT images, a vulvar tumor appears as
an area of vulvar thickening or a vulvar mass([7],[9]). On
18F-FDG PET/CT, the primary tumor may appear as an area of
focal hypermetabolism([7]).
Figure 4
FIGO stage II vulvar carcinoma. A: Axial oblique
T2WI with fat saturation, clarifying the extension to the anal
wall (arrow). The patient was treated with chemoradiotherapy and
showed no clinical evidence of disease at 6 months of follow-up.
B: Sagittal T2WI showing a vulvar tumor
extending to the lower thirds of the urethra (arrow) and vagina
(open arrow).
FIGO stage II vulvar carcinoma. A: Axial oblique
T2WI with fat saturation, clarifying the extension to the anal
wall (arrow). The patient was treated with chemoradiotherapy and
showed no clinical evidence of disease at 6 months of follow-up.
B: Sagittal T2WI showing a vulvar tumor
extending to the lower thirds of the urethra (arrow) and vagina
(open arrow).
Treatment
In stages IA, IB, and II, the first-line treatment is surgery. For stage IA
tumors, wide or radical local resection is indicated([33],[34]).
Inguinofemoral lymph node evaluation is not necessary, given the low risk of
metastases to those lymph nodes([33],[34]). For stages IB and II, the
primary treatment is determined by the tumor location. Patients with lateral
lesions (i.e., lesions located > 1 cm from the vulvar midline) should
undergo radical local resection or modified radical vulvectomy, together
with evaluation of the ipsilateral inguinal lymph node. Patients with
midline vulvar lesions should undergo radical local resection or modified
radical vulvectomy and evaluation of the right and left inguinal lymph
nodes([33],[34]). If the distal urethra or vagina is
involved, resection with clear margins is an option. If the anus is
involved, preoperative radiotherapy, with or without concurrent
chemotherapy, may be considered, in order to spare the patient a
colostomy([2]).Inguinal lymph node evaluation may be performed through sentinel node biopsy
or inguinofemoral lymph node dissection. The sentinel lymph node procedure
is recommended for unifocal tumors < 4 cm in patients without clinical or
radiological evidence of lymph node disease. Inguinofemoral lymphadenectomy
is recommended for tumors ≥ 4 cm and in cases of multifocal
disease([34]). Inguinal lymphadenectomy is indicated when
sentinel lymph nodes are not detected([33],[34]). Contralateral inguinofemoral
lymph node dissection may be performed when there is metastases to the
ipsilateral nodes([34]).The need for adjuvant therapy is determined by the nodal and primary tumor
pathology observed during surgery. In stages IA, IB, or II with negative
lymph node status, observation is usually adequate([33]). In stages IB
or II with positive lymph node status, adjuvant therapy includes external
beam radiotherapy (EBRT), with or without concurrent chemotherapy; or EBRT,
with or without concurrent chemotherapy, and completion inguinofemoral lymph
node dissection, if not previously done. If the surgical margins are
negative, observation is indicated. Conversely, if surgical margins are
close (> 8 mm) or positive, another resection should be attempted. In
cases of persistent positive margins or in patients who are not candidates
for a second resection, EBRT is recommended([33]).
Stages III and IVA-locally advanced or regional disease
Stage III vulvar carcinoma is defined as inguinal node involvement (Figure 5). When it affects one or two
inguinal lymph nodes < 5 mm or a single inguinal lymph node ≥ 5 mm, it
is classified as stage IIIA. Stage IIIB is defined as involvement of two or more
inguinal lymph nodes ≥ 5 mm or three or more < 5 mm. Stage IIIC is
defined as positive lymph nodes with extracapsular spread.
Figure 5
FIGO stage III vulvar carcinoma. Axial T2WI showing an enlarged right
inguinal lymph node (open arrow). Fine needle aspiration cytology of
the lymph node confirmed the presence of tumor cells within the
node.
FIGO stage III vulvar carcinoma. Axial T2WI showing an enlarged right
inguinal lymph node (open arrow). Fine needle aspiration cytology of
the lymph node confirmed the presence of tumor cells within the
node.Stage IVA vulvar carcinoma is defined as a tumor that is fixed to or involves the
pelvic bones; that invades the upper urethral or vaginal mucosa, bladder or
rectum; or that results in fixed or ulcerated inguinofemoral lymph nodes.
Stage IVB-metastatic disease
Stage IVB vulvar carcinoma is defined as distant metastatic disease. It involves
regional metastases to inguinal lymph nodes.On cross-sectional imaging, lymph nodes are usually considered suspicious
when the short-axis diameter is > 1 cm, which is the criterion most
commonly used for MRI. The morphology, contour, attenuation, and signal
intensity are ancillary findings that can facilitate the diagnosis of
metastatic lymph nodes. The low signal intensity of the upper two thirds of
the vagina can be disrupted by an intermediate-signal tumor on T2WI (Figure 6A), as can the normal target
appearance of the upper two thirds of the urethra (Figure 6B) and the normal high signal intensity of the
bladder or rectal mucosa (Figure 6C).
For CT, the parameters used for lymph node evaluation are the same as those
used for MRI. On 18F-FDG PET/CT, metastatic lymph nodes are FDG
avid([7]).
Figure 6
FIGO stage IVA vulvar carcinoma. A: Sagittal T2WI
with fat saturation showing involvement of the upper two thirds
of the vagina (open arrow). The patient was treated with
chemoradiotherapy and showed no clinical evidence of disease at
two years of follow-up. B: Axial T2WI showing a
central vulvar lesion with intermediate signal intensity
involving the upper two thirds of the urethra (arrow). The
patient was treated exclusively with radiotherapy, being
ineligible for concurrent chemotherapy because of a low
performance status. C: Sagittal T2WI showing a
vulvar lesion with intermediate signal intensity (arrow)
extending to the rectum (open arrow). The patient was treated
with chemoradiotherapy and had a recurrence at 27 months after
the initial diagnosis.
FIGO stage IVA vulvar carcinoma. A: Sagittal T2WI
with fat saturation showing involvement of the upper two thirds
of the vagina (open arrow). The patient was treated with
chemoradiotherapy and showed no clinical evidence of disease at
two years of follow-up. B: Axial T2WI showing a
central vulvar lesion with intermediate signal intensity
involving the upper two thirds of the urethra (arrow). The
patient was treated exclusively with radiotherapy, being
ineligible for concurrent chemotherapy because of a low
performance status. C: Sagittal T2WI showing a
vulvar lesion with intermediate signal intensity (arrow)
extending to the rectum (open arrow). The patient was treated
with chemoradiotherapy and had a recurrence at 27 months after
the initial diagnosis.All patients with stage III or IVA vulvar carcinoma should receive EBRT with
concurrent chemotherapy. Patients with imaging-positive lymph nodes should
undergo inguinofemoral dissection or FNAC([33]). If
inguinofemoral dissection reveals no metastatic lymph nodes, the patient
should receive radiotherapy covering the primary tumor (as well as the groin
in selected cases) with concurrent chemotherapy should be done. If
inguinofemoral dissection is not performed or is positive, radiotherapy with
concurrent chemotherapy, with radiotherapy coverage of the primary tumor,
groin and pelvis is indicated([33]).The need for adjuvant therapy is based on the response to chemoradiotherapy.
When there is no clinical evidence of residual tumor, observation is
recommended. Patients with residual tumor should be considered for resection
and patients with unresectable residual disease are candidates for
additional EBRT, with or without systemic therapy([33]). For distant
metastases, systemic (palliative) treatment may be considered on a
case-by-case basis([33],[34]).
FOLLOW-UP AND RECURRENT DISEASE
Recurrent disease after vulvar carcinoma is common, occurring in 25-37% of
cases([11],[35]). The most common sites of recurrence are the
perineal/vulvar region (Figure 7A), seen in
53-69% of patients with recurrence; the inguinal region (Figure 7B), seen in 19-24%; the pelvic region, seen in 6-8%;
distant sites, seen in 4-8%; and multiple locations, seen in 8-14%. Distant
metastases usually involve the following sites([11],[35]): the lung, in 45-53% of cases; the liver, in
29%; soft tissue and lymphatic systems, in 15-27%; bone (Figure 7C), in 20-25%; and the skin, in 20%.
Figure 7
Recurrence of vulvar carcinoma. A: Contrast-enhanced
sagittal CT showing extension to the floor of the bladder (arrow) at
eight years after the initial diagnosis and four years after a first
recurrence. B: Contrast-enhanced axial CT showing a
necrotic right inguinal lymph node (arrow) extending to the skin, at 8
months after modified radical vulvectomy and bilateral inguinofemoral
lymphadenectomy. C: Contrast-enhanced axial CT, with a
bone-window setting, showing lytic metastases in the sternum (arrow).
The patient was treated with chemoradiotherapy and had a recurrence at
13 months after the initial diagnosis.
Recurrence of vulvar carcinoma. A: Contrast-enhanced
sagittal CT showing extension to the floor of the bladder (arrow) at
eight years after the initial diagnosis and four years after a first
recurrence. B: Contrast-enhanced axial CT showing a
necrotic right inguinal lymph node (arrow) extending to the skin, at 8
months after modified radical vulvectomy and bilateral inguinofemoral
lymphadenectomy. C: Contrast-enhanced axial CT, with a
bone-window setting, showing lytic metastases in the sternum (arrow).
The patient was treated with chemoradiotherapy and had a recurrence at
13 months after the initial diagnosis.Isolated perineal recurrences are more common in stage I patients, whereas distant
and multiple recurrences are more common in patients with advanced-stage disease. In
addition, more than half of the isolated perineal recurrences occur in patients with
negative inguinal lymph nodes, whereas 40% of multiple recurrences occur in patients
with more than three positive lymph nodes([35]).Perineal recurrences appear with an equal distribution in the first year after
diagnosis and in the following years. Recurrences at other locations appear
predominantly within the first two years. In patients with perineal recurrence, the
5-year survival rate is 60%, being considerably lower in those with pelvic or
distant metastases. The corollary of these observations is that lymph node
metastasis is a predictor of earlier recurrence, which is considered to confer a
poorer prognosis([35]).The cornerstone of the follow-up of patients with vulvar carcinoma is history taking
and clinical examination, with careful examination of the vulva and groin regions,
because of the propensity for local recurrence([36],[37]). The use of imaging methods for the detection
of asymptomatic distant disease is of unproven benefit and is unlikely to lead to
improvements in survival, given the poor prognosis and relative ineffectiveness of
salvage therapies in patients with distant recurrence([36],[37]).The available data do not support the routine use of imaging of the groin in the
postoperative follow-up of patients with vulvar carcinoma. However, at 10-12 weeks
after definitive chemoradiotherapy, CT or 18F-FDG PET/CT should be
performed in order to document complete remission([34]).In cases of vulvar recurrence, radical local excision with inguinofemoral
lymphadenectomy is recommended, assuming that it was not previously performed or if
only sentinel node dissection was performed. The indications for adjuvant therapy
are comparable to those for the treatment of the primary
disease([33],[34]). When surgical treatment is not possible, EBRT,
with or without brachytherapy or concurrent chemotherapy, is
recommended([33]).For nodal recurrence of vulvar carcinoma, the preferred treatment is radical
excision. In radiotherapy-naïve patients, surgery may be followed by EBRT,
with or without concurrent chemotherapy. Definitive chemoradiotherapy is appropriate
when surgical treatment is not feasible([33],[34]).
CONCLUSION
Although the role of imaging methods in common gynecological malignancies is well
established, little has been published about its role in vulvar carcinoma. In brief,
MRI is useful for local and pelvic nodal staging, as well as informing the planning
of surgical interventions and radiotherapy, whereas CT or 18F-FDG PET/CT
can play an important role in the assessment of distant disease and ultrasound is
often used for image-guided biopsy procedures.
Authors: Linn Woelber; Christine Eulenburg; Matthias Choschzick; Andreas Kruell; Cordula Petersen; Friederike Gieseking; Fritz Jaenicke; Sven Mahner Journal: Int J Gynecol Cancer Date: 2012-03 Impact factor: 3.437
Authors: David E Cohn; Farrokh Dehdashti; Randall K Gibb; David G Mutch; Janet S Rader; Barry A Siegel; Thomas J Herzog Journal: Gynecol Oncol Date: 2002-04 Impact factor: 5.482